Objective: In this review article, we’ll explore TPE’s role in treating Stiff Person Syndrome(SPS) and its outcomes.

Background: Rigidity, stiffness, and intermittent spasms of the axial and extremities muscles are symptoms of Stiff Person Syndrome (SPS), a rare immune-mediated neuromuscular illness. There is no consensus on Therapeutic plasma exchange (TPE) function in SPS. Most SPS patients treated with TPE report benefits, according to recent evidence obtained from case reports and case series. In its most recent guidelines, the American Society for Apheresis does not strongly recommend TPE in SPS (Grade 2C, category III).

Method: A systematic literature search of PubMed and Embase was done to find relevant published publications that met the predetermined criteria using appropriate keyword combinations till February 1, 2023. Descriptive information about SPS patients’ demographics, diagnoses, TPE treatment, and outcomes was created using extracted data.

Results: Twenty-four studies with 81 patients underwent TPE. Major of them had classical SPS. Glutamic acid decarboxylase (GAD) autoantibodies were present in majority of the patients. Varying scoring systems were used to assess the outcome, and 50(61.72%) SPS patient showed significant improvement, 27(33.33%) showed no improvement and 4(4.93%) patients showed worsening of symptoms. The TPE complications reported in some studies were six catheter-related (bloodstream infection, thrombosis, hemorrhage) and two cases of hypotension.

Conclusion: TPE may be a safe, effective therapeutic option for patients, primarily those with severe refractory SPS, that may significantly increase mobility and reduce disease-related disability.

References: 1. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice – evidence-based approach from the writing committee of the American society for apheresis: the eighth special issue. J Clin Apher. 2019;34:171–354. doi: 10.1002/jca.21705.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/therapeutic-plasma-exchange-in-the-management-and-outcome-of-stiff-person-syndrome-a-systematic-review/